Types and Causes of Paget’s Disease | Endocrinology

Paget's Disease

What is Paget’s disease?

Paget’s disease of bone is a chronic illness of the skeleton. In healthy bones, a process called reshaping removes old pieces of bone and replaces them with fresh, new bone. It causes this process to become unbalanced, resulting in abnormally shaped, weak, and brittle new bone. It most often affects older people and occurs in about 2 to 3 percent of the population over 55 years of age.

Many patients with Paget’s disease have no symptoms and are not aware that they have the disease until X-rays are taken for some other reason. When there is bone pain and other symptoms, they may be related to the disease itself or to complications that arise from the disease, such as arthritis, bone deformity, and fractures.

In most cases, the treatment of Paget’s disease involves taking medications to help slow or stop the progress of the disease. For patients who have difficulties, surgery may be desirable to realign deformed bones or to help fractures heal.

Symptoms

Paget’s disease causes no symptoms in most people with the condition and is often seen incidentally when X-ray tests are obtained for other reasons. However, It can cause pain, deformity, fracture, and arthritis in the bones. The bone pain of Paget’s disease is localized to the affected area of the bone.

The bones most commonly affected by Paget’s disease include the spine, thigh bone (femur), pelvis, skull, clavicle (clavicle), and upper arm bone (humerus). Fractures can occur because the affected bone is not as strong as normal bone. Arthritis occurs due to deformity of the affected bone adjacent to the joint and bowing of the affected long bones. The resulting type of arthritis is osteoarthritis.

The symptoms of Paget’s disease rest on the bones affected and the severity of the disease. Enlarged bones can pinch adjacent nerves, causing tingling and numbness. Arching of the legs may occur. Hip or knee involvement can cause arthritis, lameness, as well as pain and stiffness in the hip or knee.

Headache, vision, and hearing loss can occur when the bones of the skull are affected. With widespread Paget’s disease, it is possible to develop congestive heart failure due to an increased workload on the heart. Sometimes the skin over the affected bone is warmer than usual. This is due to the increased blood supply to the pagetic bone.

Types

  • Monostotic type: When a single site of the bone is affected by Paget’s disease, it is known as the monostotic type.
  • Polyostotic type: When Paget’s disease affects multiple sites in the bone, it is called the polyostotic type.

Causes

Researchers have yet to definitively establish the cause of Paget’s disease. It appears to be hereditary. Rendering to the American College of Rheumatology, an additional than one family member has the disorder in 30 percent of cases.

Another suggestion is that the disorder is possibly due to an infection with the measles virus during childhood. Recent studies affirm that measles could alter the mechanism of bone formation and cause Paget’s disease.

Evidence suggests that the number of people with Paget’s disease has been declining over the past 25 years. Some scientists have linked the increase in vaccination in many countries and the resulting decrease in the number of people with measles to decreasing rates of Paget’s disease.

Risk factors

Years: People over the age of 40 are more likely to develop Paget’s disease of bone.

Sex: Men are affected more often than women.

National origin: Paget’s disease of bone is most common in England, Scotland, central Europe, and Greece, as well as in countries colonized by European immigrants. It is rare in Scandinavia and Asia.

Family history: If you have a close relative who has Paget’s disease of bone, they are more likely to develop the condition.

Diagnosis

During the physical exam, your doctor will examine the areas of your body that are causing you pain. You can also order X-rays and blood tests that can help confirm the diagnosis of Paget’s disease of bone.

Imaging tests

The bone changes common to Paget’s disease of bone can be revealed by:

  • X-rays: The first sign of Paget’s disease of the bone is repeatedly abnormalities found on x-rays done for other reasons. X-ray images of your bones can show areas of bone resorption, bone enlargement, and deformities that are individual of Paget’s disease of bone, such as prostration of the long bones.
  • Bone scan: In a bone scan, a radioactive substance is injected into your body. This material travels to the points in the bones most affected by Paget’s disease of bone, which is why it lights up in the scanned images.

Lab tests

People who have Paget’s disease of bone generally have elevated levels of alkaline phosphatase in their blood, which can be revealed by a blood test.

Treatment

If you don’t have symptoms, you may not need treatment. However, if the disease is active, designated by an elevated alkaline phosphatase level, and is affecting high-risk sites in your body, such as your skull or spine, your doctor may recommend treatment to prevent complications, even if you have no symptoms.

Medicines

Medications for osteoporosis (bisphosphonates) are the most common treatment for Paget’s disease of bone. Some bisphosphonates are taken by mouth, while others are injected. Oral bisphosphonates are generally well-tolerated but can irritate the gastrointestinal tract. Examples include:

  • Alendronate (Fosamax)
  • Ibandronate (Boniva)
  • Pamidronate (Aredia)
  • Risedronate (Actonel)
  • Zoledronic acid (Zometa, Reclast)

In rare cases, bisphosphonate therapy has been related to severe muscle, joint, or bone pain, which may not resolve when the drug is stopped. Bisphosphonates can also increase the risk of a rare condition in which a section of the jaw dies and deteriorates (osteonecrosis of the jaw), usually associated with active dental disease or oral surgery.

If you cannot tolerate bisphosphonates, your doctor may prescribe calcitonin (Miacalcin), a natural hormone involved in the regulation of calcium and bone metabolism. Calcitonin is a medicine that you give yourself by injection or nasal spray. Side effects can include nausea, facial flushing, and irritation at the injection site.

Surgery

In rare cases, surgery may be required to:

  • Helps heal fractures
  • Replace joints damaged by severe arthritis
  • Realign deformed bones
  • Reduce pressure on nerves

Paget’s disease of bone frequently causes the body to make too many blood vessels in pretentious bones, increasing the risk of serious blood loss during an operation.

If you are arranged for surgery involving bones affected by Paget’s disease, your physician may prescribe medications to reduce the activity of the disease, which can help reduce blood loss during surgery.

Complications

Bone expansion: The bone affected by Paget’s disease expands and can become deformed. Long bones can curve, so, for example, one leg may end up shorter than the other.

Fractures: The bone affected by Paget’s disease is weaker than usual, so it is more likely to break than healthy bone. It can also take longer than normal for a bone to heal after a fracture.

Nerve compression: When bones expand, they can sometimes squeeze nearby nerves. This is more likely to occur in the spine, which can lead to weakness and tingling in the legs.

Deafness: If the bones in the head are affected by Paget’s disease, it can lead to hearing loss, for example, if the bones around the ear become thicker.

Osteoarthritis: Abnormal bone growth can put extra pressure on the joints and damage the cartilage that covers the ends of the bones. This can lead to osteoarthritis in the joint. Symptoms of osteoarthritis include pain (especially when the joint is moved), stiffness, and sometimes swelling.

Tumors: Very rarely, a cancerous tumor can develop in a bone affected by Paget’s disease. This is estimated to happen in less than 1 in 500 cases. The first signs of this are increased pain and swelling around the tumor.

Prevention

To reduce the risk of complications associated with Paget’s disease of bone, try these tips:

  • Avoid falls, Paget’s disease of bone puts you at high risk for bone fractures
  • Protect your home from falls
  • Eat well
  • Exercise regularly
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